肝脾T细胞淋巴瘤伴乙型病毒性肝炎后肝硬化1例并文献复习

目的:提高对肝脾T细胞淋巴瘤(HSTCL)的认识。方法:报道1例HSTCL伴乙型病毒性肝炎后肝硬化,并对HSTCL的病因、临床表现、病理学与免疫表型特征、诊断及治疗进行文献复习。结果:HSTCL的病因不明,表现为发热、全血细胞减少,肝脾肿大,肝功能异常;病理示瘤细胞弥漫浸润肝/脾脏红髓血窦、骨髓血窦;免疫表型分析示瘤细胞表达CD2(+)、CD3(+)、CD7(+/-),CD4(-)、CD5(-)、CD8(-)、CD57(+/-),TCRγδ或αβ阳性,常表达NK相关抗原CD16、CD56,多表达细胞毒性颗粒蛋白TIA-1,而粒酶B、穿孔素常为阴性;PCR可检测出TCR基因重排;可有7q等臂染色体异常。联合化疗效果差,造血干细胞移植、移植前后运用2-deoxycoformycin,CD52单抗的运用等只在个例显示出良好疗效;脾切除的远期疗效不佳。结论:HSTCL临床罕见,特征性的肝、脾、骨髓窦内瘤细胞浸润,结合免疫表型检测,PCR检测出TCR基因重排等有重要的诊断与鉴别诊断价值。缺乏有效的治疗手段,预后差。

Hepatosplenic T-cell Lymphoma Complicated Viral Hepatitis Type B Secondary to Cirrhosis:A Case Report and Literature Review

Objective To improve the knowledge of hepatosplenic T-cell lymphoma(HSTCL) in clinical.Methods One case of HSTCL complocated with viral hepatitis type B secondary to cirrhosis was reported and its etiological factor,clinical manifestation,pathologic features and immunophenotype,diagnosis and therapy were analyzed by literature review.Results The etiology of HSTCL was unknown,the clinical manifestation were fever,pancytopenia,splenohepatomegalia,liver disfunction;the pathological features were lymphoma cells diffuse infiltrating red pulp sinusoid of liver or spleen,bone marrow sinusoid;the immunophenotypic expression were CD2+,CD3+,CD7+/-,CD4-,CD5-,CD8-,CD57+/-,with either γδ or αβ T cell receptor expression,NK-cell antigen CD16+、CD56+ expressed frequently;TIA-1 positive expression,perforin and granzyme B negative expression;TCR generearrangement could be detected by PCR and isochromosome 7q was a characteristic cytogenetic abnormality in HSTCL.The curative effect of combined chemotherapy was poor.The application of haemopoietic stem cell transplantation,2-deoxycoformycin,CD52 were showed good curative effect just in few cases.The long term effect of splenectomy was poor.Conclusion HSTCL was a rare disease in clinical.The characteristic manifestation of lymphoma cells diffuse infiltrating sinusoid of liver spleen and bone marrow,typical immunophenotype and TCR genemonoclonal rearrangements may be helpful in diagnosis of HSTCL,but new therapeutic strategies will be needed.